Amyotrophic
Lateral Sclerosis (ALS)

What is amyotrophic lateral sclerosis (ALS)?

ALS is a disease that causes gradual loss of control and
weakness of muscles. The mind is usually not affected,
despite worsening weakness of the body.

ALS is also called Lou Gehrig's disease, after a
well-known baseball player who died of ALS. It is rare.
About 1 new case per 100,000 people is diagnosed each year.
Symptoms most often appear in people who are between 40 and
70 years old. The symptoms worsen more rapidly in some
people than in others, eventually leading to death.

How does it occur?

ALS affects the nerves in your brain and spinal cord that
control your muscles. These nerves break down and disappear.
Your muscles then become weak and waste away because the
nerves that stimulated them are gone.

The cause of ALS is not known. It seems to run in some
families. Also, some ethnic groups, such as Pacific
Islanders, appear to be at greater risk. However, ALS most
often occurs in people who have no family history of the
disease.

What are the symptoms?

The symptoms are similar to other chronic (long-lasting)
conditions affecting the central nervous system (brain and
spinal cord). You may have:

muscle cramps and twitching

weakness in your hands and painless difficulty with
small tasks, such as fastening buttons, turning a key,
or opening a door

The disease begins slowly, most often affecting just one
limb, such as a hand, at first. As time passes, more limbs
and muscle areas become affected.

As your disability increases, you become more dependent
on others. Eventually you may be able to move only your
eyes. To others it may look like you are in a coma, but you
usually remain fully conscious and aware of your
surroundings. You are still able to see, hear, taste, smell,
and recognize touch. Bowel and bladder control and function
are usually not affected. You may become too weak to breathe
without the help of a ventilator. Respiratory failure,
pneumonia, or blood clots in the lungs may cause death.

How is it diagnosed?

There is no specific test for ALS. Your health care
provider will examine you, take your medical history, and
order tests of your brain and nervous system. An
electromyogram (EMG) may be done to test your muscles and
nerves. Other tests done to rule out other diseases may
include a computed tomography (CT) scan, magnetic resonance
imaging (MRI) scan, muscle and nerve biopsies, and blood
tests.

How is it treated?

There is no cure for this disease. Researchers are
developing and testing new drugs that they hope will improve
the treatment of ALS.

During the early stages, treatment focuses on quality of
life. Successfully managing this disease will allow you to
live the fullest life possible. Depending on your condition,
braces, walkers or a wheelchair may help. The home may need
changes to accommodate lost mobility. There are drugs that
help control drooling and spastic movement. Physical therapy
may slow the loss of muscle tone. A feeding tube may be
needed if swallowing problems become severe.

As the disease progresses, you may need to stay in a
nursing home if you cannot be cared for at home.

Discuss with your family, friends, and health care
providers how much should be done to maintain your life
during advanced stages of the disease. What medical measures
do you want to be taken at various stages of the disease? Do
you want feeding tubes and ventilators to be used? Many
people fear becoming unable to move, eat, or communicate at
the end of life and may choose to limit life-sustaining
treatments. These are difficult decisions. You and your
family may want the help of clergy or other counselors to
help you with these discussions.

What can I do to help myself?

Take care of yourself. For example:

Maintain good emotional health with support from
your family, friends, and clergy.

Maintain a healthy lifestyle, which includes eating
a balanced diet and getting regular exercise for as long
as possible.